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The importance of nomenclature for congenital cardiac disease: implications for research and evaluation

  • Matthew J. Strickland (a1) (a2), Tiffany J. Riehle-Colarusso (a1), Jeffrey P. Jacobs (a3), Mark D. Reller (a4), William T. Mahle (a5), Lorenzo D. Botto (a6), Paige E. Tolbert (a2), Marshall L. Jacobs (a7), Francois G. Lacour-Gayet (a8), Christo I. Tchervenkov (a9), Constantine Mavroudis (a10) and Adolfo Correa (a1)
  • DOI:
  • Published online: 01 December 2008

Administrative databases are often used for congenital cardiac disease research and evaluation, with little validation of the accuracy of the diagnostic codes.


Metropolitan Atlanta Congenital Defects Program surveillance records were reviewed and classified using a version of the International Pediatric and Congenital Cardiac Code. Using this clinical nomenclature as the referent, we report the sensitivity and false positive fraction (1 – positive predictive value) of the International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis codes for tetralogy of Fallot, transposition of the great arteries, and hypoplastic left heart syndrome.


We identified 4918 infants and foetuses with congenital cardiac disease from the surveillance records. Using only the International Classification of Diseases diagnosis codes, there were 280 records with tetralogy, 317 records with transposition, and 192 records with hypoplastic left heart syndrome. Based on the International Pediatric and Congenital Cardiac Code, 330 records were classified as tetralogy, 163 records as transposition, and 179 records as hypoplastic left heart syndrome. The sensitivity of International Classification of Diseases diagnosis codes was 83% for tetralogy, 100% for transposition, and 95% for hypoplastic left heart syndrome. The false positive fraction was 2% for tetralogy, 49% for transposition, and 11% for hypoplastic left heart syndrome.


Analyses based on International Classification of Diseases diagnosis codes may have substantial misclassification of congenital heart disease. Isolating the major defect is difficult, and certain codes do not differentiate between variants that are clinically and developmentally different.

Corresponding author
Correspondence to: Matthew J. Strickland, Centers for Disease Control and Prevention, 1600 Clifton Rd. NE, Mailstop E-86 Atlanta, GA 30333, United States of America. Tel: 404-421-3183; Fax: 404-498-3040; E-mail:
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